A case of double cystic esophageal duplication in VACTERL syndrome: the first case report and a review of the literature.

Background: An esophageal duplication cyst (EDC) is a rare malformation resulting from the embryonic foregut. VACTERL syndrome is a genetic disorder affecting many systems of the human body. We report the first case of VACTERL syndrome associated to asymptomatic double EDC. Case report: A girl with anorectal malformation and rectovestibular fistula, kidney malformation, and various vertebral defects came to our attention at the time of birth. VACTERL disease was diagnosed. She underwent Peña anoplasty at 4 months of life without complications. MRI was conducted at the age of 2. It accidentally showed a double esophageal duplication (12 mm × 35 mm × 10 mm) at the D7-D9 level. We planned a thoracoscopy; previous intraoperative esophagogastroduodenoscopy showed an external compression of the native esophagus. Two duplicated esophageal lesions were removed. The patient made an uneventful recovery and was completely asymptomatic at long-term follow-up. Conclusions: VACTERL syndrome is still a not well-defined disease. Based on the current literature, this is the first case of a double esophageal duplication in a patient affected by VACTERL syndrome. According to us, the thoracoscopic approach of esophageal duplications can be followed by experts. Complete surgical excision is possible even if the cyst shares a common muscular wall with the esophagus. For this reason, we suggest to close the muscular wall by a simple interrupted suture.

Double isolated asynchronous duodenal perforation due to abdominal blunt trauma in a child: A case report.

BACKGROUND: Isolated duodenal perforation following blunt abdominal trauma is a rare injury in children. Bicycle accidents (falling on to the handlebar) are a frequent cause of blunt abdominal trauma in children and may occasionally be associated with isolated duodenal perforation (IDP). Prompt diagnosis and surgical treatment are vital to prevent increased morbidity and mortality. CASE PRESENTATION: We report the rare case of an 11-year-old boy admitted for blunt abdominal trauma and treated for an asynchronous double IDP. The first perforation, located on the 2nd/3rd portion of the duodenum, was promptly diagnosed by contrast-enhanced abdominal CT scan after a negative US scan, five hours after injury, and the lesion repaired with a single stitch suture. The second duodenal perforation appeared in the duodenal bulb as a worsening biliary leakage, 48 h after the primary suture of the initial lesion. The perforation was initially seen by digestive endoscopy and sutured in the same way as the first lesion. A third laparotomy was needed 4 days later due to an intestinal obstruction, after which the patient was recovered completely and was discharged home. DISCUSSION AND CONCLUSION: IDP is a rare consequence of blunt abdominal trauma, and is normally associated with a lesion of other organs, such as the pancreas or bile duct. A delayed diagnosis strongly increases the incidence of morbidity and mortality, and different kinds of surgical management have been proposed, depending on the type of lesion. To our knowledge, this is the first case described in literature of a double isolated asynchronous duodenal perforation following blunt abdominal trauma in children.

Vesicoureteral reflux in infants: what do we know about the gender prevalence by age?

OBJECTIVE: Vesicoureteral reflux (VUR) affects up to 1% of Caucasian children. Primary VUR is characterized by failure of the ureterovesicular junction to prevent urine from traveling in a retrograde fashion from the bladder to the ureters and the kidneys. Several reports in the literature describe the prevalence of this condition in pediatric patients; overall, VUR affects more males during infancy and with higher grades. However, a thorough consideration of these articles reveals important contradictions regarding the prevalence by gender and age. We analyzed those contradictions and suggested a possible explanation based on our single center experience with this patient group. In particular, for the age interval 0-2 years: we have found that (1) VUR mostly affects boys; (2) the male/female ratio steadily declines over time; (3) the unequal prevalence between males and females essentially disappears when children reach the age of two years. CONCLUSIONS: The natural history of VUR in infant boys differs from that of infant girls, and therefore requires a gender-specific approach. Available data support the need to redefine the categorization and clinical guidelines for this disease.

A rare association of inlet patch with laryngospasm: a report of two children and literature review.

The inlet patch (IP) is an area of heterotopic gastric mucosa in the cervical esophagus commonly seen during upper endoscopy. Although generally asymptomatic IP has been associated with esophageal and supraesophageal symptoms and, though rare, in adult with malignant transformation. We describe two cases of recurrent episodes of laryngospasm associated with IP. In both cases there was a good response to prolonged acid suppression therapy.

One trocar assisted pyeloplasty (OTAP): initial experience and codification of a technique.

BACKGROUND: The Anderson-Hynes pyeloplasty is still the gold standard for the surgical treatment of the ureteropelvic junction obstruction (UPJO) in children. To reduce the surgical invasivity, laparoscopic (RPSc) and retroperitoneoscopic (RPSc) pyeloplasty have been proposed but concerns have been expressed. In the RPSc approach, the small operative space and the technical difficulties have limited the application and diffusion of this techinique while in case of laparoscopy, a transperitoneal technique is used to treat a retroperitoneal disease. We report our initial experience with the "one trocar assisted pyeloplasty" (OTAP) in the treatment of UPJO in children. METHODS: From January 2005 to December 2006 sixteen children (13 males--3 females) from 2 to 18 months of age (mean age 5.6 months), with impaired monolateral renal function underwent surgical treatment for UPJO. In 11 cases the obstruction was on the left side and in one case a crossing vessel was found. The renal pelvis was anteriorly reached using a 10 mm operative telescope via a flank 12 mm incision. The UPJ was exteriorized and a dismembered pyeloplasty performed also for the crossing vessel. In two cases a 6 Fr uretero-pyelo-nephrostomy catheter was used. RESULTS: The patients were discharged after four days without complications. The cosmetic results are excellent. In one case, a small perianastomotic leakage was seen. The ultrasonographic follow-up demonstrated the spontaneous resolution of the leakage. The post operative US follow-up showed the reduction of the dilatation in all cases. DISCUSSION: This is an initial experience and a longer follow-up is needed. However, in our opinion the OTAP could be considered as a safe and feasible alternative to the laparoscopic or retroperitoneoscopic pyeloplasty, especially in very small children and also in presence of crossing vessels.

The role of emergency thoracoscopy in the management of congenital cystic adenomatoid malformation of the lung associated with oesophageal atresia.

Congenital cystic adenomatoid malformation of the lung associated with oesophageal atresia is exceptional. The authors describe a case of a mixed type I - II congenital cystic adenomatoid malformation of the left lung associated with oesophageal atresia and tracheooesophageal fistula in a male infant. The interesting aspect of this case is not only the extremely rare association - only two reports in the literature - but the surgical choices. In fact, two weeks after surgical repair of the oesophageal atresia, the growth of the cystic volume of the congenital adenomatoid malformation made respiratory weaning very difficult, and it was therefore decided to solve the respiratory distress by opening the tensional cysts using a thoracoscopic access. The advantage of this treatment was that it decompressed the underlying healthy lung tissue and permitted the expansion of the normal lobar parenchyma. This is a palliative approach that allows the mandatory definitive resection of the affected lung lobe to be postponed until a later time.

Long-term outcome of laparoscopic Nissen and Toupet fundoplication in normal and neurologically impaired children.

BACKGROUND: Laparoscopic fundoplication is a commonly performed procedure in children. This report describes the incidence of long-term recurrence and complications after laparoscopic Nissen or Toupet fundoplication in neurologically impaired and normal children. METHODS: Fifty-three children operated on before 1999 were reviewed. All children were evaluated clinically and with a barium meal study thereafter. Symptomatic children and those with abnormal barium meal underwent 24 h pH monitoring. RESULTS: A total of 45 patients were included in the study. The mean follow-up was 4.5 years. All, except one asymptomatic child that declined, had a barium meal. Four were abnormal (2 parahiatal hernias and 2 slight episodes of reflux). Four patients had symptoms related to the operation and 2 to clinical recurrence. Only 1 asymptomatic child with slight reflux at barium meal revealed abnormal 24 h pH monitoring. Finally, 6.6% patients were found to have late recurrence (2 clinical and 1 pHmetry). There was an obvious increase in children's weight, especially in neurologically impaired patients. CONCLUSION: Laparoscopic antireflux surgery is of value in children with gastroesophageal reflux disease. The long-term results are comparable with open surgery, and there was no difference in term of wrap failure between neurologically impaired and normal children.

Blue rubber bleb nevus syndrome and gastrointestinal haemorrhage: which treatment?

PURPOSE: To describe a paediatric case of "Blue Rubber Bleb Nevus Syndrome" (BRBNS) or Bean's syndrome, a rare systemic disorder characterised by cutaneous and gastrointestinal vascular malformations that often lead to overt life-threatening gastrointestinal bleeding or occult blood loss with severe anaemia and iron deficiency. CASE REPORT: A 6-year-old girl with multiple characteristic cutaneous vascular lesions was admitted for a massive rectal bleeding. A few months previously she was endoscopically treated for gastric angiomas which developed into melaena. Preoperative investigations revealed the recurrence of gastric lesions. At laparotomy, more than 25 angiomas of the GI tract were found. Multiple intestinal resections were carried out. RESULTS: No intraoperative or postoperative problems occurred and the girl is completely healthy without further bleeding after a follow-up period of three years. CONCLUSIONS: BRBNS belongs to the group of vascular venous malformations. Most of the time it occurs sporadically, but it can be inherited as an autosomal dominant trait. Recent analysis identified a locus on chromosome 9 responsible for venous malformations. BRBNS patients present typical skin lesions, with some lesions having a rubber-like nipple appearance; the number of skin and GI lesions and the severity of anaemia are correlated. Treatment is dependent on the extent of gut involvement and the severity of the clinical picture. In the absence of massive bleeding, a conservative treatment will be sufficient; otherwise resections are mandatory, but additional lesions may subsequently develop. Management with electrocautery or laser photocoagulation are usually not effective even if some reports recommend them. Pharmacological treatment is useless. Prognosis of BRBNS is unknown.

Transumbilical laparoscopic-assisted appendectomy (TULAA): a safe and useful alternative for uncomplicated appendicitis.

AIM: To compare transumbilical laparoscopic-assisted appendectomy (TULAA) and open appendectomy (OA) in cases of acute uncomplicated appendicitis. METHODS: 58 TULAA and 65 OA in patients with a preoperative diagnosis of acute uncomplicated appendicitis are compared. Pneumoperitoneum was obtained with a transumbilical 10 mm trocar (telescope access) and a 5 mm operative channel introduced in the left iliac fossa. Appendectomy was performed outside the abdomen, after the exteriorisation of the appendix through the transumbilical incision. RESULTS: In the TULAA group, operative time and hospital stay were reduced. Conversion was necessary in one case (1.7 %), and in one case (1.7 %), an additional 5 mm operative channel was introduced. Neither intra- or postoperative complications were found in the TULAA group, with excellent cosmetic results. In the OA group we had a wound infection (1.5 %) and in 8 cases (12.3 %) an enlargement of the incision was necessary. CONCLUSIONS: TULAA is the best approach in uncomplicated appendicitis. It is less invasive and traumatic, permits a complete evaluation of the peritoneal cavity with superior cosmetic results, especially in obese patients and in cases of ectopic appendicitis.

The renal-resistive index from the last 3 months of pregnancy to 6 months old.

OBJECTIVE: To measure the renal resistive index (RI, an estimate of renal vascular resistance, used to assess upper tract obstructive uropathy) from the last trimester of pregnancy to the sixth month of life in a large series of healthy subjects, and thus to identify normal values that can be used routinely. During the first semester of life significant haemodynamic changes during the physiological development of the kidney cause considerable variability in RI, which is thus considered less reliable in this period. SUBJECTS AND METHODS: From September 1998 to October 1999, 93 subjects (186 renal units, RU) were enrolled; 32 were fetuses in the last trimester of pregnancy (group 1, 64 RU) and 61 were children (122 RU), 30 aged 0-1 month (group 2, 60 RU), 20 aged 1-3 months (group 3, 40 RU) and 11 aged 3-6 months (group 4, 22 RU). All subjects underwent colour Doppler ultrasonography and the RI of the renal artery was measured for each kidney. RESULTS: The RI was very high in group 1 but decreased noticeably during the first 6 months of life, reaching values similar to those in adults after the third month. The variability in RI continuously declined with age, becoming less important. The normal ranges for groups 1-4 were 0.67-0.88, 0.57-0.90, 0.60-0.84 and 0.65-0.75, respectively. There were no statistically significant differences between the left and right kidneys. CONCLUSION: In the first semester of life there is more than one landmark value of RI depending on the month of age of the infant. This should be considered when assessing upper tract obstructive uropathies after birth and the RI should be compared with the normal ranges reported herein.

Conservative treatment of neonatal primary megaureter.

The growing agreement toward the conservative treatment of primary megaureter (PM) is supported by the increasingly frequent reports in the literature of spontaneous resolution of this pathology after few years of attendance. If the PM is asymptomatic without the presence of parenchymatous damage, and the diuretic scintiscan does not show a definite obstruction of the uretero-vesical junction, the conservative treatment should be the choice for the neonatal forms of PM. We report our experience of 14 neonatal PM, conservatively managed out of a total of 22 PM observed between 1990 and 1996. All the patients have been controlled with serial ultrasonography and Tc99 DTPA scintigraphy. Three of them underwent a surgical operation because of persistence or impairment of the clinical and scintigraphic pictures after a 12-18 months' follow-up. Some of the remainders are completely recovered while others are going toward resolution. The conservative treatment of neonatal PM is therefore confirmed to be sure and effective, and in spite of the different attitudes expressed by reliable authors in the up-to-date literature we believe it should be undertaken for the asymptomatic forms in which there is no documented uretero-vesical obstruction.